Mycobacterium avium Complex Infection in a Patient with Sickle Cell Disease and Severe Iron Overload

A 34-year-old female with sickle cell anemia (hemoglobin SS disease) and severe iron overload presented to our institution with the subacute presentation of recurrent pain crisis, fever of unknown origin, pancytopenia, and weight loss. A CT scan demonstrated both lung and liver nodules concerning fo...

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Main Authors: Kamal Shemisa, Nasima Jafferjee, David Thomas, Gretta Jacobs, Howard J. Meyerson
Format: Article
Language:English
Published: Wiley 2014-01-01
Series:Case Reports in Infectious Diseases
Online Access:http://dx.doi.org/10.1155/2014/405323
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author Kamal Shemisa
Nasima Jafferjee
David Thomas
Gretta Jacobs
Howard J. Meyerson
author_facet Kamal Shemisa
Nasima Jafferjee
David Thomas
Gretta Jacobs
Howard J. Meyerson
author_sort Kamal Shemisa
collection DOAJ
description A 34-year-old female with sickle cell anemia (hemoglobin SS disease) and severe iron overload presented to our institution with the subacute presentation of recurrent pain crisis, fever of unknown origin, pancytopenia, and weight loss. A CT scan demonstrated both lung and liver nodules concerning for granulomatous disease. Subsequent biopsies of the liver and bone marrow confirmed the presence of noncaseating granulomas and blood cultures isolated Mycobacterium avium complex MAC. Disseminated MAC is considered an opportunistic infection typically diagnosed in the immunocompromised and rarely in immunocompetent patients. An appreciable number of mycobacterial infection cases have been reported in sickle cell disease patients without immune dysfunction. It has been reported that iron overload is known to increase the risk for mycobacterial infection in vitro and in vivo studies. While iron overload is primarily known to cause end organ dysfunction, the clinical relationship with sickle cell disease and disseminated MAC infection has not been reported. Clinical iron overload is a common condition diagnosed in the sub-Saharan African population. High dietary iron, genetic defects in iron trafficking, as well as hemoglobinopathy are believed to be the etiologies for iron overload in this region. Patients with iron overload in this region were 17-fold more likely to die from Mycobacterium tuberculosis. Both experimental and clinical evidence suggest a possible link to iron overload and mycobacterial infections; however larger observational studies are necessary to determine true causality.
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spelling doaj-art-ef742828508649598f546b6c4bc0458a2025-02-03T05:44:04ZengWileyCase Reports in Infectious Diseases2090-66252090-66332014-01-01201410.1155/2014/405323405323Mycobacterium avium Complex Infection in a Patient with Sickle Cell Disease and Severe Iron OverloadKamal Shemisa0Nasima Jafferjee1David Thomas2Gretta Jacobs3Howard J. Meyerson4University of Texas Southwestern University Hospital, 5323 Harry Hines Boulevard, Dallas, TX 75390, USAYale University Hospital, 20 York Street, New Haven, CT 06510, USAUniversity Hospitals Case Western Reserve University, 11100 Euclid Avenue, Cleveland, OH 44106, USAUniversity Hospitals Case Western Reserve University, 11100 Euclid Avenue, Cleveland, OH 44106, USAUniversity Hospitals Case Western Reserve University, 11100 Euclid Avenue, Cleveland, OH 44106, USAA 34-year-old female with sickle cell anemia (hemoglobin SS disease) and severe iron overload presented to our institution with the subacute presentation of recurrent pain crisis, fever of unknown origin, pancytopenia, and weight loss. A CT scan demonstrated both lung and liver nodules concerning for granulomatous disease. Subsequent biopsies of the liver and bone marrow confirmed the presence of noncaseating granulomas and blood cultures isolated Mycobacterium avium complex MAC. Disseminated MAC is considered an opportunistic infection typically diagnosed in the immunocompromised and rarely in immunocompetent patients. An appreciable number of mycobacterial infection cases have been reported in sickle cell disease patients without immune dysfunction. It has been reported that iron overload is known to increase the risk for mycobacterial infection in vitro and in vivo studies. While iron overload is primarily known to cause end organ dysfunction, the clinical relationship with sickle cell disease and disseminated MAC infection has not been reported. Clinical iron overload is a common condition diagnosed in the sub-Saharan African population. High dietary iron, genetic defects in iron trafficking, as well as hemoglobinopathy are believed to be the etiologies for iron overload in this region. Patients with iron overload in this region were 17-fold more likely to die from Mycobacterium tuberculosis. Both experimental and clinical evidence suggest a possible link to iron overload and mycobacterial infections; however larger observational studies are necessary to determine true causality.http://dx.doi.org/10.1155/2014/405323
spellingShingle Kamal Shemisa
Nasima Jafferjee
David Thomas
Gretta Jacobs
Howard J. Meyerson
Mycobacterium avium Complex Infection in a Patient with Sickle Cell Disease and Severe Iron Overload
Case Reports in Infectious Diseases
title Mycobacterium avium Complex Infection in a Patient with Sickle Cell Disease and Severe Iron Overload
title_full Mycobacterium avium Complex Infection in a Patient with Sickle Cell Disease and Severe Iron Overload
title_fullStr Mycobacterium avium Complex Infection in a Patient with Sickle Cell Disease and Severe Iron Overload
title_full_unstemmed Mycobacterium avium Complex Infection in a Patient with Sickle Cell Disease and Severe Iron Overload
title_short Mycobacterium avium Complex Infection in a Patient with Sickle Cell Disease and Severe Iron Overload
title_sort mycobacterium avium complex infection in a patient with sickle cell disease and severe iron overload
url http://dx.doi.org/10.1155/2014/405323
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AT davidthomas mycobacteriumaviumcomplexinfectioninapatientwithsicklecelldiseaseandsevereironoverload
AT grettajacobs mycobacteriumaviumcomplexinfectioninapatientwithsicklecelldiseaseandsevereironoverload
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